**1. Introduction**

Autoimmune diseases occur due to the response of the adaptive immune system to self-antigens and mediators as well as tissue damage, leading to breaking self-tolerance. Autoimmune diseases can be divided into two groups. The first group particularly involves an organ, such as type 1 diabetes mellitus (T1DM) and thyroiditis. The second group, however, presents itself in the form of systemic complications [1]. Systemic disorders affect some organs and tissues such as the eye and optic nerve, skin, and joints. Systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and multiple sclerosis (MS) are good examples of autoimmune diseases with multiorgan involvement [2, 3].

SLE is a long-lasting, febrile, proinflammatory multiorgan disease of the connective tissue, primarily involving the skin, joints, and serosal membranes [2, 4]. Moreover, SLE is associated with various diseases including neurological conditions (stroke, seizure, cranial neuropathy, and cognitive dysfunction), serositis, leukopenia, thrombocytopenia, antiphospholipid syndrome, lymphadenopathy, autoimmune hemolytic anemia, fever, arthritis, Livedo reticularis, renal disease, Raynaud's phenomenon, oral ulcer, and malar rash [5]. The disease often affects women, Black, Hispanic, and Asian populations (ten times more frequently in women compared to men).

NPSLE or central nervous system (CNS) lupus denotes the condition where lupus influences the brain, spinal cord, and other nerves. Interestingly, SLE can affect the nervous system as neurological (N) and psychiatric (P) syndromes that are known as neuropsychiatric SLE (NPSLE) [6, 7]. NPSLE could be found in 40% of all people who have SLE. The prevalence and incidence of SLE vary based on the region, sex distribution, ethnicity, and socioeconomic factors. For example, the incidence rate of SLE in the US Medicare population varies between 3.7 and 49.0 in 100,000 person-year and the prevalence rate varies from 48 to 366.6 in 100,000 person-year. But in Europe, the incidence rate of SLE ranges from 1.5 to 7.4 per 100,000 person-year and the prevalence rate ranges from 29.3 to 210 (Global incidence rate ranges from 1.5 to 11 per 100,000 person-year and the prevalence rate varies between 13 and 7,773.5 per 100,000 person-year). It is expected that the mortality rate will decrease in the future [8]. By contrast, several essays show an increase in case numbers [9, 10].

To understand and increase information about the pathology, etiology, and treatment of this disease, animal models are used. Several articles on the pathology of NPSLE have been carried out in various aspects, such as genetic model design, complement system, cytokine involvement, and auto-antibody against brain antigens [11]. NPSLE leads to disruption and augmented the penetrability of the blood-brain barrier (BBB). Environmental factors (e.g., viruses such as Epstein-Barr virus, smoking, vitamin D level, air pollution, and medication drugs) affect the neuroimmunopathogenesis of SLE [12, 13].

Scientists have been facing many research difficulties within the field of SLE. In lupus, the inflammation happens due to the attack of the immune system against tissues and organs. Yet, the precise details of mechanisms underlying SLE are unclear. Diagnosing NPSLE is difficult because clinicians have to rule out other causes, including tumors and infections. Current treatments aim to inhibit the excessive activities of the immune system and prevent organ failure. Drugs used for patients depend on the symptoms that appear [14].

The present chapter rapidly reviews recent research into the clinical presentation, molecular mechanisms, diagnosis, management, and treatment of SLE with a focus on NPSLE. Finally, our discussion offers novel insights into the role of Immunoinformatics in future clinical research.

*Lupus and the Nervous System: A Neuroimmunoloigcal Update on Pathogenesis and Management… DOI: http://dx.doi.org/10.5772/intechopen.107970*
